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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Congenital extrahepatic portosystemic shunt
Definitions
- Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
- Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
- Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
- Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
- Type 2: Intact but hypoplastic intrahepatic PV with extrahepatic portosystemic shunt
- Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
- Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Baiges A et al: Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 71(2):658-69, 2020
- Bueno J et al: Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts. J Pediatr Surg. 55(9):1897-902, 2020
- DiPaola F et al: Congenital portosystemic shunts in children: associations, complications, and outcomes. Dig Dis Sci. 65(4):1239-51, 2020
- Rajeswaran S et al: Abernethy malformations: evaluation and management of congenital portosystemic shunts. J Vasc Interv Radiol. 31(5):788-94, 2020
- Sokollik C et al: Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 56(6):675-81, 2013
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Congenital extrahepatic portosystemic shunt
Definitions
- Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
- Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
- Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
- Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
- Type 2: Intact but hypoplastic intrahepatic PV with extrahepatic portosystemic shunt
- Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
- Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Baiges A et al: Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 71(2):658-69, 2020
- Bueno J et al: Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts. J Pediatr Surg. 55(9):1897-902, 2020
- DiPaola F et al: Congenital portosystemic shunts in children: associations, complications, and outcomes. Dig Dis Sci. 65(4):1239-51, 2020
- Rajeswaran S et al: Abernethy malformations: evaluation and management of congenital portosystemic shunts. J Vasc Interv Radiol. 31(5):788-94, 2020
- Sokollik C et al: Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 56(6):675-81, 2013
